Pediatric Mixed Connective Tissue Disease

Curr Rheumatol Rep. 2016 May;18(5):28. doi: 10.1007/s11926-016-0576-x.


Pediatric-onset mixed connective tissue disease is among the rare disease entities in pediatric rheumatology and includes features of arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus, and systemic sclerosis. Accurate recognition and diagnosis of the disease is paramount to prevent long-term morbidity. Advances in the genetic and immunologic understanding of the factors involved in the etiopathogenesis provide an opportunity for improvements in prognostication and targeted therapy. The development of a multinational cohort of patients with mixed connective tissue disease would be invaluable to provide more updated data regarding the clinical presentation, to develop a standardized treatment approach, disease activity and outcome tools, and to provide data on long-term outcomes and comorbidities.

Keywords: Etiopathogenesis; Mixed connective tissue disease; Outcome; Pediatric; Treatment.

Publication types

  • Review

MeSH terms

  • Antirheumatic Agents / therapeutic use*
  • Child
  • Humans
  • Mixed Connective Tissue Disease / diagnosis*
  • Mixed Connective Tissue Disease / drug therapy*
  • Prognosis


  • Antirheumatic Agents